NATIONAL SOCIETY OF PEDIATRIC SURGERY OF THE REPUBLIC OF MOLDOVA

DIEULAFOY DISEASE. DIAGNOSTIC AND TREATMENT ISSUES

Hotineanu V., Iliadi A.

Public Institution State University of Medicine and Pharmacy “Nicolae Testemiţanu”,

Surgery Department no. 2, Chisinau, Republic of Moldova

ABSTRACT

This article includes the management of one clinical case. A 27 years old patient was urgently hospital-
ized with upper digestive hemorrhage diagnosis, caused by Dieulafoy disease. The primary diagnosis was
confused and needs more pre- and intraoperative confirmations. Histological and morphological examina-
tions confirmed the clinical diagnosis. The surgical treatment and intensive care contribute to removal of
vital risk and better therapeutic outcome.
Key words: Dieulafoy disease; hemorrhage; histology, treatment

INTRODUCTION •• Clinical development is characterized by:

Angiodysplasias of the gastro-intestinal tract are
vascular abnormalities situated in the wall structure
of digestive tract organs, from the esophagus to
anus. They are often acquired and, more rarely, con-
genital, clinically appreciated or latent, evolving on
their own or in association with other pathologies.
Fontch P. defines angiodisplazia as an idiopathic
syndrome consisting of ectasia of intestinal submu-
cosa, with or without ectasia of overlying mucosa
capillaries [3, 4, 6, 7, 8].
Dieulafoy disease represents an arterial an-
giodysplasia characterized by anomaly of vascular
development of the stomach submucosa, with the
presence of large arterial erosions in the absence of
vasculitis with the formation of acute ulcers, often
complicated with digestive haemorrhage [2, 4, 7].
Similar cases were described at autopsy by Gallard
(1884). The detailed clinical picture was described
by Dieulafoy G. (1998), being distinguished as a
separate nosologic form – simple ulcers [3, 4, 5].
Clinical features of the disease are:
•• It is characterized by proximal stomach lesion,
localization of ulcers on the posterior wall,
small curvature (60-80%), at a distance of up
to 6 cm from the esophagus junction [2, 7];
•• It occurs between 16-96 years (average 50-60
years), the ratio of men: women being 2:1;
•• It is presented as a source of superior diges-
tive hemorrhage (0.3-5.8%), recurrent (18-
100%), cataclysmic hemorrhages in 1/3 of pa-
tients [1, 3, 4];
sudden onset without abdominal pain; active
hemorrhage (hematemesis, melena) [2, 8];
Predisposing factors are: chronic alcoholism,
salicylates and steroids administration, stress. The
absence of ulcerative anamnesis is important [2, 8].
The Dieulafoy ulcer may be located on the way
of the digestive tract and may be associated with
other pathologies such as: gastric and duodenal ul-
cers, Mallory-Weiss syndrome, Crohn’s disease, gas-
tric cancer [2, 4, 6].
Histomorphologically it is characterized by: the
presence of large arteries in the ulcerous defect of
the stomach mucosa, dysplasia of muscular sub-
strate vessels and lymphocytic infiltration [2, 4, 6].
Diagnosis of Dieulafoy disease in the multidisci-
plinary study of causes of digestive hemorrhages is
based on endoscopic techniques (48-98%), selective
angiography results (30%) and histomorphological
study (20%). All of these methods are used to identi-
fy Dieulafoy disease, assess the localization of vascu-
lar fistula, and evaluate treatment outcomes [2, 4, 7].
Clinical case: Patient S., aged 27 years old, is
hospitalized urgently for a specific symptom of su-
perior digestive hemorrhage: repeated vomiting
with fresh blood and clots, vertigo, headache, gen-
eral weakness, melena. From the past, it is known
that in 2012 he suffered the first upper gastrointes-
tinal hemorrhage, and he was treated conservative-
ly under stationary conditions. He was healed and
discharged with the diagnosis of Mallory-Weiss syn-
drome, receiving recommendations for ambulatory
treatment.

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The general exam reveals that at the admission, The particularities of the clinical case: diagnos-
the patient is in a general serious condition. The tic and therapeutic problems with major impact on
visible skin and mucosa are pale and clean. Cold pre- and postoperative clinical development. The
sweats, hypotension – 90/60 mmHg, tachycardia, uncertain diagnosis has contributed to errors in
FCC-120 bpm /min. therapeutic conduct. The histomorphological diag-
The patient presented a hypovolemic shock. The nosis confirmed the clinical diagnosis of Dieulafoy
nasogastric tube evokes gastric content –hematem- Disease (fig. 2, 3, 4).
esis, fresh clots. Rectal exam – rectal ampoule with
changed blood content. Laboratory samples show
post hemorrhagic anemia – Hb - 70 g / l; erythro-
A
cytes – 2.4 • 1012 g /l.
The admission diagnosis focused on Mallo-
ry-Weiss syndrome complicated with superior di-
gestive hemorrhage gr. II-III; hypovolemic shock.
The diagnosis has been endoscopically confirmed.
Two flat defects of 1.8-1.3 cm and 1.5-0.3 cm, fi-
brin-coated, are determined in the esophageal junc-
tion. Emergency treatment in the intensive care unit
includes correction of volume and hematic correc-
tion, antiulcer medication, hemostatic. The patient
is in clinical and hemodynamic improvement. More
than 18 hours after hospitalization, the hemorrhage
is re-evaluated. Repeated endoscopic control is per-
formed, the source of hemorrhage cannot be appre-
ciated due to inconclusive endoscopic signs. The
Blackmore probe for the Mallory-Weiss syndrome
was applied, the upper digestive hemorrhage being
B
stopped.
For a differential diagnosis of superior diges-
tive hemorrhage with digestive angiodysplasia, re-
peated endoscopic control was performed over 48
hours after admission, attesting to the presence of 2
mucosal rupture of 2.0 cm and 1.4 cm in diameter,
which covered the gastroesophageal junction with
fibrin. In the posterior subcardial area on fornix – a
0.5 cm pitched defect with freshly fixed thrombus,
laminar hemorrhage.
Endoscopic diagnosis: Dieulafoy’s disease com-
plicated with repeated, active digestive hemor-
rhage. Surgical treatment is indicated immediately.
Intraoperative endoscopic control confirmed:
the stomach of normal size and shape, elastic walls.
In the stomach lumen – coffee grounds hemorrhage, C
the glossy pale mucosa of the stomach. Preserved
relief of the fornix, esophageal and cardiopulmonary
varicose veins were absent. In the posterior subcar-
dial area of ​​fornix, a 0.5 cm pitched defect with a
freshly fixed thrombus was assessed.
Gastric resection is practiced. In the proximal
subcardial region of the stomach, on the large cur-
vature - the posterior wall, at 3.0 cm from the esoph-
agus junction, there is a mucosal ulceration of 3.0
mm, covered with a fresh thrombus, fixed without
signs of active hemorrhage. Blood clotting in the jet
was seen in the removal of the clot. The resection
was performed in the direction of the large curvature
(fig. 1). In the gastro-splenic ligament, large vessels
were found which do not correspond to the anatom- Fig. 1. a) Endoscopic view, b) Intraoperative, c) Post-
ical realities. operative

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 NATIONAL SOCIETY OF PEDIATRIC SURGERY OF THE REPUBLIC OF MOLDOVA

DISCUSSIONS
A Gastrointestinal angiodysplasias are responsi-
ble for approximately 6% of gastrointestinal tract
hemorrhages and 1.2-8% of hemorrhages located in
the upper digestive tract. This is the fourth cause of
digestive hemorrhage after ulcer disease, varicose
hemorrhage and colon diverticulosis, all of which
cause 85% of digestive hemorrhage [1, 6, 7, 8].
Concomitant with the more frequent use of con-
temporary imaging methods (endoscopic and an-
giographic), the incidence of angiodysplasia diag-
nosis has increased, patients being included in the
category of idiopathic digestive hemorrhage.
According to the Camillieri classification (1996),
clinical, histological arterial-venous malformations
are divided into [6, 8]:
•• Type I – arteriovenous malformations with
predominance of dysplastic veins with the
small intestine / colon wall;
B •• Type II – Osler-Webber-Rendu disease;
•• Type III – haemartomatous vascular lesions
encountered in Peutz-Jeghers syndrome, blue
rubber blue nevus and Klipper-Trenaunay syn-
drome;
•• Type IV – gastrointestinal angiodysplasia,
localized predominantly on the right colon,
associated with aortic stenosis, (Heyde syn-
drome);
•• Type V – Massive gastrointestinal bleeding
caused by submucosa lesion with large vessel
(Dieulafoy Disease).

CONCLUSIONS

Dieulafoy’s disease is a rare cause of digestive

C bleeding, being identified as a cause of obscure
bleeding. Diagnosis of pathology is difficult and
complex (endoscopy, selective angiography and
histological examination). EFGDS intraoperatively
confirms clinical diagnosis. Surgical interventions
are prevalent, and it is necessary to minimize “blind”
resections.

Fig. 2. A - piece of the gastric wall in the region adja-

cent to the Dieulafoy lesion: 1) mucosa without signs
of inflammation, 2) sSubmucosa, 3) artery dilated with
blood stasis. H-E. x100; B - A piece of the gastric wall
in the region adjacent to the Dieulafoy lesion: 1) gas-
tric mucosa without signs of inflammation, 2) artery
with eroded walls, 3) hemorrhage and mucosal lesion
adjacent to the eroded vessel, 4) Dilated arteries in
submucosa. H-E. x40; C - hemorrhage outbreak with
mucosal eruption. H-E. x200

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